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Major and Minor Temporal Bone Abnormalities in Children With and Without Congenital Sensorineural Hearing Loss
John E. McClay, MD;
Richard Tandy, PhD;
Kenneth Grundfast, MD;
Sukgi Choi, MD;
Gilbert Vezina, MD;
George Zalzal, MD;
Ayal Willner, MD
Arch Otolaryngol Head Neck Surg. 2002;128:664-671.
Objective To determine the extent of correlation between sensorineural hearing
loss (SNHL) and abnormal temporal bone anatomy in children.
Design Axial and coronal high-resolution computed tomographic scans of the
temporal bones of 247 children (494 ears) aged 2 months to 15 years with and
without SNHL were blindly reviewed. The presence or absence of mild or severe
cochlear dysplasias, vestibular dysplasias, and an enlarged vestibular aqueduct
(VA) were recorded. The width of the VA was measured. The height, width, and
length of the internal auditory canal (IAC) were measured, and abnormalities
were described as narrow, widened, or bulbous. Clinical information was then
reviewed to determine the presence or absence of a congenital syndrome and/or
SNHL, and historical factors that might be responsible for SNHL.
Main Outcome Measure The relationship between radiographic findings and SNHL.
Results One hundred thirteen patients (185 ears) had SNHL. Significant abnormal
temporal bone anatomy in children with vs without SNHL included major cochlear
and vestibular dysplasias (17% vs 0%; P<.001),
enlarged VA (>2 mm) (5% vs 0%; P<.001), and narrow
IAC ( 2 mm) (4% vs 1%; P= .03). The average IAC
width (4.85 vs 5.02 mm), height (4.39 vs 4.62 mm), and length (11.22 vs 11.44
mm) were not statistically different between children with vs without SNHL.
In children with vs without SNHL, neither a widened (0.5% vs 3.6%) nor a bulbous
(9% vs 8%) IAC was seen more often in children with SNHL. In ears with SNHL,
the presence of a congenital syndrome significantly increased the risk of
cochlear and vestibular abnormalities of the temporal bone (45% vs 14%; P<.001), including IAC abnormalities (30% vs 2%;P<.001), which overall were more commonly seen in children
with (20%) vs without (3%) a congenital syndrome regardless of the presence
of SNHL. No children with an enlarged VA had a congenital syndrome.
Conclusions Well-established temporal bone abnormalities such as cochlear and vestibular
abnormalities and a grossly enlarged vestibular aqueduct are significantly
found in children with SNHL. A narrow IAC is found more often in children
with vs without SNHL. No significant correlation is found between SNHL and
radiographic findings of a widened or bulbous IAC. In children with a congenital
syndrome, more IAC abnormalities were seen, regardless of the presence of
SNHL. In children with SNHL, the presence of a congenital syndrome increases
the likelihood of a cochlear or vestibular abnormality.
From the Division of Pediatric Otolaryngology, Department of Otolaryngology/Head
and Neck Surgery, The University of Texas at Southwestern Medical Center,
Dallas (Dr McClay); the Department of Kinesiology, College of Health Sciences,
University of Nevada at Las Vegas (Dr Tandy); the Department of Otolaryngology/Head
and Neck Surgery, Boston Medical Center, Boston, Mass (Dr Grundfast); the
Departments of Pediatric Otolaryngology (Drs Choi and Zalzal) and Pediatric
Neuroradiology (Dr Vezina), Childrens National Medical Center, Washington,
DC; and the Department of Otolaryngology, Long Beach Memorial Medical Center,
Long Beach, Calif (Dr Willner).
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